ICD-10: N04.3

Nephrotic syndrome with diffuse mesangial proliferative glomerulonephritis

Additional Information

Description

Nephrotic syndrome with diffuse mesangial proliferative glomerulonephritis is classified under the ICD-10-CM code N04.3. This condition is characterized by a specific set of clinical features and underlying pathophysiology that are important for diagnosis and management.

Clinical Description

Nephrotic Syndrome Overview

Nephrotic syndrome is a kidney disorder that results in the following key symptoms:
- Proteinuria: Excessive protein in the urine, typically greater than 3.5 grams per day.
- Hypoalbuminemia: Low levels of albumin in the blood, leading to decreased oncotic pressure.
- Edema: Swelling, particularly in the legs, ankles, and around the eyes, due to fluid retention.
- Hyperlipidemia: Elevated levels of lipids in the blood, often as a compensatory response to low protein levels.

Diffuse Mesangial Proliferative Glomerulonephritis

Diffuse mesangial proliferative glomerulonephritis is a specific type of glomerulonephritis characterized by:
- Mesangial Cell Proliferation: An increase in the number of mesangial cells, which are located in the glomeruli of the kidneys. This proliferation can lead to glomerular damage and dysfunction.
- Immune Response: Often associated with immune-mediated processes, which may be triggered by infections, autoimmune diseases, or other systemic conditions.
- Histological Findings: On kidney biopsy, there is typically a diffuse increase in mesangial matrix and cellularity, which can be identified through immunofluorescence and electron microscopy.

Pathophysiology

The pathophysiology of nephrotic syndrome with diffuse mesangial proliferative glomerulonephritis involves complex interactions between immune mechanisms and kidney function. The proliferation of mesangial cells can lead to:
- Glomerular Injury: Resulting in increased permeability of the glomerular filtration barrier, allowing proteins to leak into the urine.
- Inflammation: The immune response can cause further damage to the glomeruli, exacerbating proteinuria and other symptoms.

Diagnosis

Diagnosis typically involves:
- Clinical Evaluation: Assessment of symptoms such as edema, weight gain, and fatigue.
- Laboratory Tests: Urinalysis showing significant proteinuria, serum albumin levels indicating hypoalbuminemia, and lipid profiles showing hyperlipidemia.
- Kidney Biopsy: May be performed to confirm the diagnosis and assess the extent of mesangial proliferation and other histological changes.

Treatment

Management of nephrotic syndrome with diffuse mesangial proliferative glomerulonephritis may include:
- Corticosteroids: To reduce inflammation and immune response.
- Immunosuppressive Agents: Such as cyclophosphamide or rituximab, particularly in cases resistant to steroids.
- Supportive Care: Including diuretics for edema, dietary modifications, and management of hyperlipidemia.

Conclusion

ICD-10 code N04.3 encapsulates a specific and complex condition that requires a thorough understanding of its clinical presentation, underlying mechanisms, and management strategies. Early diagnosis and appropriate treatment are crucial to improving patient outcomes and preventing complications associated with nephrotic syndrome and its underlying causes.

Clinical Information

Nephrotic syndrome with diffuse mesangial proliferative glomerulonephritis, classified under ICD-10 code N04.3, is a specific type of nephrotic syndrome characterized by a combination of clinical features and underlying pathological changes in the kidneys. This condition is often associated with various underlying causes, including infections, systemic diseases, and primary kidney disorders. Below is a detailed overview of the clinical presentation, signs, symptoms, and patient characteristics associated with this condition.

Clinical Presentation

Overview of Nephrotic Syndrome

Nephrotic syndrome is a clinical condition defined by a triad of symptoms: significant proteinuria, hypoalbuminemia, and edema. In the case of diffuse mesangial proliferative glomerulonephritis, these symptoms are accompanied by specific histological changes in the kidney's glomeruli, characterized by mesangial cell proliferation and expansion of the mesangial matrix.

Signs and Symptoms

  1. Edema:
    - Patients typically present with generalized edema, which may be more pronounced in the lower extremities and can progress to anasarca (severe generalized edema) due to fluid retention[1].

  2. Proteinuria:
    - A hallmark of nephrotic syndrome, patients often exhibit significant proteinuria, typically greater than 3.5 grams per day. This is usually detected through urine dipstick tests or 24-hour urine collection[2].

  3. Hypoalbuminemia:
    - Due to the loss of protein in urine, serum albumin levels are often low, leading to hypoalbuminemia. This can contribute to the development of edema and ascites[3].

  4. Hyperlipidemia:
    - Patients may also present with elevated lipid levels, particularly cholesterol and triglycerides, as the liver compensates for low serum albumin levels by increasing lipoprotein synthesis[4].

  5. Fatigue and Weakness:
    - General malaise, fatigue, and weakness are common complaints among patients, often due to fluid overload and metabolic disturbances[5].

  6. Hypertension:
    - Some patients may develop hypertension, which can complicate the clinical picture and management of the syndrome[6].

Patient Characteristics

  • Age:
  • Nephrotic syndrome can occur at any age but is more prevalent in children, particularly those between the ages of 2 and 6 years. However, adults can also be affected, often presenting with different underlying causes[7].

  • Gender:

  • There is a slight male predominance in pediatric cases, while adult cases may show a more equal distribution between genders[8].

  • Underlying Conditions:

  • Patients with diffuse mesangial proliferative glomerulonephritis may have associated systemic conditions such as lupus erythematosus, infections (e.g., hepatitis B or C), or may be idiopathic in nature. A thorough evaluation for secondary causes is essential[9].

  • Family History:

  • A family history of kidney disease may be present, particularly in cases of genetic predisposition to glomerular diseases[10].

Conclusion

Nephrotic syndrome with diffuse mesangial proliferative glomerulonephritis presents with a distinct set of clinical features, including significant edema, proteinuria, hypoalbuminemia, and hyperlipidemia. Understanding the signs and symptoms, along with patient characteristics, is crucial for timely diagnosis and management. Clinicians should consider both primary and secondary causes of nephrotic syndrome when evaluating patients, as this can significantly influence treatment strategies and outcomes. Regular monitoring and supportive care are essential components of managing this complex condition.

Approximate Synonyms

Nephrotic syndrome with diffuse mesangial proliferative glomerulonephritis, classified under ICD-10 code N04.3, is a specific type of kidney disorder characterized by a combination of nephrotic syndrome and a particular form of glomerulonephritis. Understanding the alternative names and related terms for this condition can enhance clarity in medical documentation and communication. Below are some of the relevant terms associated with this diagnosis.

Alternative Names

  1. Diffuse Mesangial Proliferative Glomerulonephritis: This is the primary condition associated with the nephrotic syndrome in this ICD-10 code. It refers to the proliferation of mesangial cells in the glomeruli of the kidneys.

  2. Nephrotic Syndrome: While this is a broader term, it is essential to note that nephrotic syndrome encompasses various underlying causes, including glomerulonephritis.

  3. Membranoproliferative Glomerulonephritis (MPGN): Although not identical, MPGN can sometimes be associated with diffuse mesangial proliferation and may be used interchangeably in some contexts.

  4. Primary Nephrotic Syndrome: This term may be used when the nephrotic syndrome is not secondary to other diseases, although it is less specific than N04.3.

  5. Secondary Nephrotic Syndrome: This term can apply if the nephrotic syndrome is a result of another underlying condition, such as systemic diseases or infections.

  1. Glomerular Diseases: This is a broader category that includes various types of kidney diseases affecting the glomeruli, including nephrotic syndrome and glomerulonephritis.

  2. Kidney Disease: A general term that encompasses all forms of kidney dysfunction, including nephrotic syndrome and its various etiologies.

  3. Proteinuria: A common symptom of nephrotic syndrome, referring to the presence of excess protein in the urine, which is a hallmark of this condition.

  4. Edema: This term describes the swelling that occurs due to fluid retention, commonly seen in patients with nephrotic syndrome.

  5. Hypoalbuminemia: A condition characterized by low levels of albumin in the blood, often associated with nephrotic syndrome.

  6. Hypertension: High blood pressure can be a related condition in patients with nephrotic syndrome, although it is not a defining feature.

  7. Chronic Kidney Disease (CKD): While nephrotic syndrome can lead to CKD, this term refers to a broader spectrum of kidney dysfunction.

Conclusion

Understanding the alternative names and related terms for ICD-10 code N04.3 is crucial for accurate diagnosis, treatment, and communication among healthcare providers. These terms not only help in identifying the specific condition but also in recognizing its implications and associated symptoms. For healthcare professionals, using precise terminology ensures clarity in patient records and enhances the quality of care provided.

Treatment Guidelines

Nephrotic syndrome with diffuse mesangial proliferative glomerulonephritis, classified under ICD-10 code N04.3, is a complex condition characterized by significant proteinuria, hypoalbuminemia, and edema, often resulting from underlying glomerular diseases. The management of this condition typically involves a combination of pharmacological treatments, dietary modifications, and supportive care. Below is a detailed overview of standard treatment approaches for this specific diagnosis.

Pharmacological Treatments

1. Corticosteroids

Corticosteroids are often the first line of treatment for nephrotic syndrome, particularly in cases associated with glomerulonephritis. Prednisone is commonly prescribed to reduce inflammation and proteinuria. The dosage and duration depend on the severity of the condition and the patient's response to therapy. A typical regimen may start with high doses, tapering down as the patient's condition improves[2].

2. Immunosuppressive Agents

In cases where corticosteroids are ineffective or if the patient experiences frequent relapses, additional immunosuppressive agents may be introduced. Commonly used medications include:
- Cyclophosphamide: Often used in conjunction with corticosteroids for its immunosuppressive properties.
- Calcineurin Inhibitors: Drugs like cyclosporine or tacrolimus can be effective in reducing proteinuria and managing nephrotic syndrome.
- Mycophenolate Mofetil: This agent may also be considered, particularly in patients who cannot tolerate other immunosuppressants[1][3].

3. Diuretics

Diuretics are essential for managing edema associated with nephrotic syndrome. Loop diuretics, such as furosemide, are commonly used to promote fluid excretion and alleviate swelling. The choice of diuretic and its dosage will depend on the patient's fluid status and kidney function[1].

4. Angiotensin-Converting Enzyme (ACE) Inhibitors

ACE inhibitors, such as lisinopril, are beneficial in managing hypertension and reducing proteinuria. They work by decreasing intraglomerular pressure, which can help protect kidney function over time[2].

5. Statins

Statins may be prescribed to manage dyslipidemia, which is common in nephrotic syndrome due to increased lipid synthesis in response to low serum albumin levels. They help reduce cardiovascular risk associated with hyperlipidemia[1].

Dietary Modifications

1. Sodium Restriction

A low-sodium diet is often recommended to help control edema and hypertension. Reducing sodium intake can minimize fluid retention and improve overall fluid balance[3].

2. Protein Intake

Dietary protein management is crucial. While patients may need to increase protein intake to compensate for losses due to nephrotic syndrome, the amount should be carefully monitored to avoid excessive strain on the kidneys. A consultation with a dietitian can help tailor dietary needs based on individual circumstances[1].

Supportive Care

1. Monitoring and Follow-Up

Regular follow-up appointments are essential to monitor kidney function, proteinuria levels, and the effectiveness of treatment. Adjustments to therapy may be necessary based on the patient's response and any side effects experienced[2].

2. Patient Education

Educating patients about their condition, treatment options, and lifestyle modifications is vital for effective management. Patients should be informed about the signs of complications, such as infections or thromboembolic events, which can occur due to the hypercoagulable state associated with nephrotic syndrome[3].

Conclusion

The management of nephrotic syndrome with diffuse mesangial proliferative glomerulonephritis (ICD-10 code N04.3) requires a comprehensive approach that includes pharmacological treatment, dietary adjustments, and ongoing monitoring. Corticosteroids and immunosuppressive agents form the cornerstone of therapy, while supportive measures help manage symptoms and prevent complications. Regular follow-up and patient education are crucial to ensure optimal outcomes and improve the quality of life for affected individuals.

Diagnostic Criteria

Nephrotic syndrome with diffuse mesangial proliferative glomerulonephritis, classified under ICD-10 code N04.3, is a specific condition characterized by a combination of nephrotic syndrome symptoms and the histological findings of diffuse mesangial proliferation in the kidneys. The diagnosis of this condition involves several criteria, which can be categorized into clinical, laboratory, and histopathological assessments.

Clinical Criteria

  1. Symptoms of Nephrotic Syndrome: The primary clinical features include:
    - Proteinuria: Typically greater than 3.5 grams per day, indicating significant protein loss in urine.
    - Edema: Swelling, particularly in the legs, ankles, and around the eyes, due to fluid retention.
    - Hypoalbuminemia: Low levels of albumin in the blood, often below 3 g/dL, resulting from protein loss.
    - Hyperlipidemia: Elevated levels of lipids in the blood, which can occur as a compensatory response to low albumin levels.

  2. History and Physical Examination: A thorough medical history and physical examination are essential to rule out other causes of nephrotic syndrome and to assess the severity of symptoms.

Laboratory Criteria

  1. Urinalysis:
    - Dipstick Test: A positive test for protein, often showing 3+ or 4+ proteinuria.
    - 24-Hour Urine Collection: Quantitative measurement confirming proteinuria exceeding 3.5 grams per day.

  2. Blood Tests:
    - Serum Albumin: Measurement of serum albumin levels, typically showing hypoalbuminemia.
    - Lipid Profile: Assessment of cholesterol and triglyceride levels, which are usually elevated.

  3. Kidney Function Tests:
    - Serum Creatinine: Evaluation of kidney function, as nephrotic syndrome can lead to renal impairment.

Histopathological Criteria

  1. Kidney Biopsy: A definitive diagnosis often requires a kidney biopsy to assess the underlying pathology. The biopsy findings for diffuse mesangial proliferative glomerulonephritis typically include:
    - Mesangial Cell Proliferation: Increased number of mesangial cells in the glomeruli.
    - Immunofluorescence Microscopy: May show IgG and complement deposition in the mesangial area, which is characteristic of this type of glomerulonephritis.
    - Electron Microscopy: Can reveal changes in the glomerular structure, such as electron-dense deposits in the mesangial region.

Differential Diagnosis

It is crucial to differentiate nephrotic syndrome with diffuse mesangial proliferative glomerulonephritis from other types of nephrotic syndrome and glomerulonephritis. Conditions such as minimal change disease, focal segmental glomerulosclerosis, and other forms of glomerulonephritis must be considered based on clinical presentation and biopsy results.

Conclusion

The diagnosis of nephrotic syndrome with diffuse mesangial proliferative glomerulonephritis (ICD-10 code N04.3) relies on a combination of clinical symptoms, laboratory findings, and histopathological evidence. A comprehensive approach is essential to ensure accurate diagnosis and appropriate management of the condition. If you have further questions or need additional information, feel free to ask!

Related Information

Description

  • Proteinuria in urine exceeds 3.5 grams per day
  • Low levels of albumin in the blood
  • Swelling due to fluid retention
  • Elevated lipid levels in the blood
  • Mesangial cell proliferation in kidneys
  • Immune response triggers glomerular damage
  • Kidney biopsy confirms diagnosis and damage

Clinical Information

  • Significant proteinuria is present
  • Generalized edema occurs frequently
  • Hypoalbuminemia leads to fluid retention
  • Hyperlipidemia often accompanies nephrotic syndrome
  • Fatigue and weakness are common complaints
  • Hypertension can complicate the condition
  • Age range varies from children to adults
  • Male predominance in pediatric cases

Approximate Synonyms

  • Diffuse Mesangial Proliferative Glomerulonephritis
  • Nephrotic Syndrome
  • Membranoproliferative Glomerulonephritis (MPGN)
  • Primary Nephrotic Syndrome
  • Secondary Nephrotic Syndrome
  • Glomerular Diseases
  • Kidney Disease
  • Proteinuria
  • Edema
  • Hypoalbuminemia

Treatment Guidelines

  • Corticosteroids reduce inflammation and proteinuria
  • Immunosuppressive agents used in resistant cases
  • Loop diuretics manage edema and fluid status
  • ACE inhibitors control hypertension and proteinuria
  • Statins manage dyslipidemia and cardiovascular risk
  • Sodium restriction controls edema and hypertension
  • Protein intake carefully monitored to avoid kidney strain

Diagnostic Criteria

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